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MCM > Carbohydrate Metabolism > Flashcards

Carbohydrate Metabolism Flashcards

1
Q

Francoi-Bickel Syndromes

A

Mutation in Glut2

Unable to take up glucose fructose galactose

Failure to thrive
Hepatomegaly
Fasting hyperglycemia

Treat with vit d and phosphate.

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2
Q

RATE LIMITING ENZYME IN GLYCOLYSIS

A

PFK-1

ACTIVATED BY AMP, F26BP, High insulin dephospho pfk2 (jmakes PFK 2 active)

Inhibited by ATP, Citrate, Glucagon (activates protein kinase A which phosphorylation PFK 2 to decrease PFK1 activity

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3
Q

Protein Kinase

A

Activated by f1 by and insulin

Inhibited by atp alanine glucagon (cAMP activates PKA phosphorylation)

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4
Q

Hexokinase,Glucokinase

A

Hexo- High affinity so any glucose will activate it

GK- Low affinity for glucose
Regulated by GK-RP (sequesters GK to nucleus) most active when glucose levels are high

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5
Q

Tarui Disease

A

GSD 7

Defective PFK-1

Excercised-induced muscle crams
Hemolytic anemia
High bilirubin and jaundice

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6
Q

Von Gierke Disease

A

GSD 1

Deficient in glucose 6 phosphotase

Marked fasting hypoglycemia

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MCM (3 decks)

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